Mirizzi syndrome refers to an uncommon phenomenon that results from extrinsic compression of an extrahepatic biliary duct from one or more calculi within the cystic duct or gallbladder. It is a functional hepatic syndrome but can often present with biliary duct dilatation and can mimic other hepatobiliary pathologies such as cholangiocarcinoma 2.
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Epidemiology
The syndrome occurs in approximately 1 in every 1000 patients with gallstones 8.
Risk factors
A low insertion of the cystic duct into the common bile duct as well as a tortuous cystic duct are thought to be risk factors.
Clinical presentation
Patients may present with recurrent episodes of jaundice and cholangitis. It can be associated with acute cholecystitis.
Pathology
Classification
Management decisions depend on the type of Mirizzi syndrome 6,9 .
Multiple classification systems have been proposed 11:
Csendes classification:
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type I: extrinsic compression of the common hepatic duct (CHD)
type Ia: by impacted gallstone in the gallbladder neck or cystic duct
type Ib: if cystic duct is absent 7
type II: erosion of CHD wall and formation of cholecystocholedochal fistula (up to one-third CHD wall circumference is involved)
type III: up to two-thirds of CHD wall circumference is involved in a cholecystocholedochal fistula
type IV: entire CHD wall is involved in a cholecystocholedochal fistula
type V: any of the above with cholecystoenteric fistula 6
Beltrán classification:
type I: external compression of the bile duct
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type II: cholecystobiliary fistula
type IIa: less than 50% the diameter of the bile duct
type IIb: greater than 50% the diameter of the bile duct
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type III: cholecystobiliary fistula and cholecystoenteric fistula
type IIIa: without gallstone ileus
type IIIb: with gallstone ileus
Radiographic features
Fluoroscopy
On ERCP, the stricture is smooth and often concave to the right ref.
CT
The gallbladder wall may be diffusely thickened and may enhance with contrast ref.
MRI
MRCP classically shows a large impacted gallstone in the gallbladder neck or cystic duct, or signs of inflamed gallbladder causing proximal dilatation of the extra and intrahepatic biliary tree, with distal gradual tapering of the extrahepatic biliary duct caliber to the site of obstruction ref.
Treatment and prognosis
Complications
Prolonged biliary obstruction and inflammation may lead to:
cholecystobiliary fistula: leads to choledocholithiasis
gallstone ileus 6: if the stone passes into the small bowel through a cholecystoenteric fistula
secondary biliary cirrhosis 10
delayed onset biliary stricture 10
History and etymology
Kehr first described the syndrome in 1905, and in 1908 Ruge provided a more detailed description of the condition, stating that there was jaundice associated with extrinsic compression of the bile ducts 11. An Argentinian surgeon Pablo Luis Mirizzi (1893-1964), described the syndrome in a paper from 1940, although 1948 is often quoted, as in this year he published a paper in which it became widely-known 4,6. Mirizzi performed the first operative cholangiogram in 1931. He was named a Master Surgeon (Cirujano Maestro) in 1956 by the Argentinian Surgeons Society (Sociedad Argentina de Cirugía) 6.
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